A single-center case series of eight patients with the rare plasma cell dyscrasia of acquired Fanconi syndrome secondary to monoclonal gammopathy
A single-center case series of eight patients with the rare plasma cell dyscrasia of acquired Fanconi syndrome secondary to monoclonal gammopathy
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DOI：
10.3109/10428194.2015.1025392
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发表时间：
2015-11-02
影响因子：
2.6
通讯作者：
Zhuang, Junling
中科院分区：
文献类型：
--
作者：
Liu, Yang;Zhu, Tienan;Zhuang, Junling
获得性范可尼综合征是一种罕见的单克隆丙种球蛋白病。我们回顾性总结了2007年1月至2014年4月在北京协和医院（北京协和医院）收治的8例继发于单克隆丙种球蛋白病的FS患者的病例。所有患者均有近端肾小管功能的广泛或部分损害。6例患者被诊断为意义不明的单克隆丙种球蛋白病（MGUS），2例被诊断为多发性骨髓瘤（MM）。尽管对2例MM患者和1例MGUS患者进行了化疗，但副蛋白水平的降低并未导致代谢异常的改善。所有患者均接受了持续的营养补充，导致骨痛明显缓解，生活质量改善。虽然大多数患者的肾功能逐渐下降，但在平均31个月的随访时间内，没有患者发生终末期肾病（ESRD），也没有MGUS病例转化为MM。
Acquired Fanconi syndrome (FS) is a rare presentation of monoclonal gammopathy. We retrospectively summarized the cases of eight patients with FS secondary to monoclonal gammopathy at Peking Union Medical College Hospital (PUMCH) from January 2007 to April 2014. All patients had generalized or partial impairment of proximal renal tubular function. Six patients were diagnosed with monoclonal gammopathy of undetermined significance (MGUS), and two were diagnosed with multiple myeloma (MM). Although chemotherapy was administered to the two patients with MM and to one patient with MGUS, decreased paraprotein levels did not lead to improvements in metabolic abnormalities. All patients received continuous supplementation with deficient nutrients, leading to marked remission of bone pain and improved quality of life. Although renal function in most patients gradually declined, none of the patients developed end-stage renal disease (ESRD) during an average follow-up time of 31 months, and no case of MGUS has yet transformed into MM.